Clinical Relevance Of The Endothelial Cell Neoplasm Model

Criticisms related to the transformed status of EOMA cells are tempered by the fact that any results obtained with this model have direct clinical applicability. Hemangioendotheliomas (HE) are vascular neoplasms of borderline/intermediate malignancy.32 They do not metastasize; however, in humans, the mortality rate ranges from 12 to 24%.33 This lesion is also associated with the development of Kassabach— Memtt syndrome, a consumptive coagulopathy with a 20 to 30% mortality rate.34'35 The fact that mice injected with EOMA cells develop Kassabach—Merritt syndrome is a good indicator of how closely this model mimics the human condition.22 It may be argued that HE is not a common entity, but the true incidence cannot be determined because it is frequently misdiagnosed or mistakenly referred to as a hemangioma.3436-38 The HE lesion has several key characteristics in common with hemangiomas:1 they proliferate rapidly during the first year of life;2 they express cytokines and growth factors to facilitate proliferation; and3 they have the potential to involute. The fact that HE and hemangiomas share these key characteristics significantly enhances the probability that results obtained with the EOMA model can be extrapolated to hemangiomas, which are the most common soft-tissue tumors in infants and children.

The overall incidence of endothelial cell neoplasms in children is approximately 10%, and although many of these lesions eventually involute, they have serious or life-threatening complications in 5 to 10% of affected children, and 50% of all lesions leave permanent residual deformity.39 Because of the high-risk side-effect profiles of current treatment regimens—long-term high-dose steroids or interferon-alpha—many children and their parents are forced to accept a period of deformity, as shown in Figure 16.2, until involution occurs. These therapies are usually reserved for patients with life-, limb-, or vision-threatening complications, so a need for less toxic diet-based treatments is acute.

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